Successful Treatment with Rituximab in a Patient with Clinically Suspected Small-vessel Primary Central Nervous System Vasculitis Refractory to Cyclophosphamide Pulse Therapy
Abstract
A 58-year-old man developed a progressive cognitive impairment and recurrent cerebral infarctions that were refractory to antithrombotic therapy. No abnormalities were detected in the major cerebral arteries, and a cerebrospinal fluid analysis revealed mild inflammatory findings. Although a brain biopsy was not feasible, the patient was clinically diagnosed with small-vessel primary central nervous system vasculitis based on comprehensive evaluations. Treatment with glucocorticoids and cyclophosphamide failed to prevent a relapse. The presence of cerebrospinal fluid oligoclonal bands and elevated interleukin-6 levels suggested intrathecal humoral or lymphocyte-mediated immune activity, and rituximab therapy led to clinical improvement. This clinical improvement indirectly suggests B-cell involvement, consistent with the efficacy of rituximab. Rituximab may therefore be an option for treating cyclophosphamide-resistant PCNSV.
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BibTeX
@article{okamoto2026successful,
title = {{Successful Treatment with Rituximab in a Patient with Clinically Suspected Small-vessel Primary Central Nervous System Vasculitis Refractory to Cyclophosphamide Pulse Therapy}},
author = {Takuya Okamoto and Kazuki Yokokawa and Bungo Hirose and Naotoshi Iwahara and Syuuichirou Suzuki and Shin Hisahara},
journal = {Internal Medicine},
year = {2026},
month = sep,
doi = {10.2169/internalmedicine.7758-26},
url = {https://www.jstage.jst.go.jp/article/internalmedicine/advpub/0/advpub_7758-26/_article},
note = {CC BY-NC-ND},
}
RIS
TY - JOUR
TI - Successful Treatment with Rituximab in a Patient with Clinically Suspected Small-vessel Primary Central Nervous System Vasculitis Refractory to Cyclophosphamide Pulse Therapy
AU - Takuya Okamoto
AU - Kazuki Yokokawa
AU - Bungo Hirose
AU - Naotoshi Iwahara
AU - Syuuichirou Suzuki
AU - Shin Hisahara
PY - 2026
DA - 2026/09/29
JO - Internal Medicine
DO - 10.2169/internalmedicine.7758-26
UR - https://www.jstage.jst.go.jp/article/internalmedicine/advpub/0/advpub_7758-26/_article
AB - A 58-year-old man developed a progressive cognitive impairment and recurrent cerebral infarctions that were refractory to antithrombotic therapy. No abnormalities were detected in the major cerebral arteries, and a cerebrospinal fluid analysis revealed mild inflammatory findings. Although a brain biopsy was not feasible, the patient was clinically diagnosed with small-vessel primary central nervous system vasculitis based on comprehensive evaluations. Treatment with glucocorticoids and cyclophosphamide failed to prevent a relapse. The presence of cerebrospinal fluid oligoclonal bands and elevated interleukin-6 levels suggested intrathecal humoral or lymphocyte-mediated immune activity, and rituximab therapy led to clinical improvement. This clinical improvement indirectly suggests B-cell involvement, consistent with the efficacy of rituximab. Rituximab may therefore be an option for treating cyclophosphamide-resistant PCNSV.
SN - 1349-7235
SN - 0918-2918
ER -